Zusammenfassung
Klinishi-pathologische, ultrastrukturelle und immunhistochemische Befunde von einem Fall der IgG1 Schwerkettenkrankheit werden detailliert beschrieben. Dem Protein fehlt die VH und CH1 Region; die Sequenz beginnt mit dem 225 Rest. Pathologisch-anatomisch befand sich vor allem eine plasmazytische B-Zellgeschwulst der Lympknoten vom Typus eines immunblastischen Sarkoms. Es bestanden neoplastische Infiltrate auch in anderen Organen, darunter dem Knochenmark. Die Ultrastruktur der Geschwulstzellen war durch mehr oder weniger reiches plasmazellähnliches endoplasmatisches Retikulum charakterisiert, häufig mit eigentümlichen konzentrischen Formationen. Die immunhistochemische Untersuchung erwies die Produktion eines abnormalen Proteins in den neoplastischen Zellen verschiedentlichen Differenzierungsgrades. Von diesem sowie von den anderen Fällen der Literatur ergibt sich, daß die γ HCD von der Proliferation und von Tumoren des lympho-plasmazytären Zellsystems verursacht wird, während die Retikulumzellen dabei keine Bedeutung besitzen.
Summary
Clinicopathological ultrastructural, and immunohistochemical findings of a new case of IgG1 heavy chain disease are reported in detail. The abnormal protein lacks the VH and CH1 region with sequence starting at 225 residue. The main pathologic feature was a plasmacytic tumor of the lymph nodes with B-cell immunoblastic sarcoma patterns. Neoplastic diffusion to other organswas also present. Plasmacytic neoplastic cells have also been found in the bone marrow. The ultrastructure of the neoplastic cells was characterized by more or less abundant plasmacytic-like endoplasmic reticulum with very frequent peculiar whorled configurations. Immunohistochemical methods revealed the abnormal protein production by neoplastic cells in different stages of differentiation. From this case and from the data of the literature it is concluded that the γHCD is due to a neoplastic proliferation of lymphoplasmacytic cells whereas the reticulum cells are never involved.
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References
Adlersberg, J.B., Grann, V., Zucker-Franklin, D., Frangione, B., Franklin, E.C.: An unusual case of a plasma cell neoplasm with an IgG 3γ myeloma and a γ 3 heavy chain disease protein. Blood 51, 85–96 (1978)
Benbassat, J., Fluman, N., Zlotnick, A.: Monoclonal immunoglobulin disorders: a report of 154 cases. Am. J. Med. Sci. 271, 325–334 (1976)
Bloch, K.J., Lee, L., Mills, J.A., Haber, E.: Gamma heavy chain disease. An expanding clinical and laboratory spectrum. Am. J. Med. 55, 61–70 (1973)
Buxbaum, J.N.: Heavy chain diseases in man. La Ricerca Clin. Lab. 6, 301–317 (1976)
Carbonara, A.O., Paolino, W., Malavasi, F., Stramignoni, A., de Lange, G., van Loghem, E., Franklin, E.C.: A new case of gamma heavy chain disease: clinical immunochemical and structural characterization. Vox Sang. 37, 89–95 (1979)
Castleman, B. et al.: Case records of Massachusetts General Hospital. N. Engl. J. Med. 283, 1332–1339 (1970)
Dammacco, F., Rigoli, E., Ferrarese, M., Bonomo, L.: Gamma heavy chain disease in a young girl. Haematologica 61, 278–290 (1976)
Delmas-Marsalet, Y., Voisin, D., Hennache, G., Bauters, F., Goudemand, M.: Étude clinique et biologique de la maladie des chaínes lourdes gamma. A propos d'une nouvelle observation. Nouv. Rev. Franç. Hemat. 11, 717–734 (1971)
Denk, H., Radaszkiewicz, T., Witting, Ch.: Immunofluorescence studies on pathologie routine material: application to malignant lymphomas. Beitr. Pathol. 159, 219–225 (1976)
Ellman, L.L., Bloch, K.J.: Heavy-chain disease: report of a seventh case. N. Engl. J. Med. 278, 1195–1201 (1968)
Faguet, G.B., Barton, B.P., Smith, L.L., Garver, F.A.: Gamma heavy chain disease: clinical aspects and characterization of a deleted noncovalently linked γ l heavy chain dimer (BAZ). Blood 49, 495–505 (1977)
Fisher, E.R., Zawadzki, Z.A.: Ultrastructural features of plasma cells in patients with paraproteinemias. Am. J. Clin. Pathol. 54, 779–789 (1970)
Franklin, E.C., Lowenstein, J., Bigelow, B., Meltzer, M.: Heavy Chain Disease. A new disorder of serum γ-globulins. Report of the first case. Am. J. Med. 37, 332–350 (1964)
Frizzera, G., Long, J.C., Berard, C.W.: Evolution of angio-immunoblastic lymphadenopathy. N. Engl. J. Med. 297, 59–60 (1977)
Gallart, M.T., Canals, J., Cañadell, E., Cortez, L., Moragas, A., Schwartz, S.: A new case of gammaheavy chain disease. Acta Haematol. 59,262–276 (1978)
Kaiserling, E.: Non-Hodgkin-Lymphome. Ultrastruktur and Cytogenese. Veröffentlichungen aus der Pathologie. Progress in Pathology 105. Stuttgart, New York: Fischer 1977
Keller, H., Spengler, G.A., Skvaril, F., Flury, W., Noseda, G., Riva, G.: Zur Frage der “heavy chain disease”. Ein Fall von IgG-Heavy-Chain-Fragment und IgM typ K. Paraproteinämie mit Plasmazellenleukämie. Helv. Med. Acta. (Suppl.) 50, 133–134 (1970)
Kim, H., Heller, P., Rappaport H.: Monoclonal gammopathies associated with lymphoproliferative disorders: A morphologic study. Am. J. Clin. Pathol. 59, 282–294 (1973)
Lebreton, J.P., Rivat, C., Rivat, L., Guillemot, L., Ropartz, C.: Une immunoglobulinopathie méconnue: la maladie des chaines lourdes. Presse Méd. 75, 2251–2254 (1967)
Lindholm, L., Westin, J., Weinfeld, A., Eyrich, R.: Heavy chain disease. En preliminar studie av tva fall. Nord. Med. 84, 1573–1573 (1970), quoted by Gallart et al. (1978)
Loyau, G., Barré, J.P., L'Hirondel, J.L., Laniéce, M., Preud-Homme, J.L.: Maladie des chaines lourdes gamma. A propos d'une nouvelle observation. Nouv. presse méd. 4, 957–959 (1975)
Luft, J.H.: Improvements in epoxy resin embedding methods. J. Biophys. Biochem. Cytol. 9, 409–414 (1961)
Lyons, R.M., Chaplin, H., Tillack, T.W., Majerus, P.W.: Gamma heavy chain disease: Rapid, sustained response to cyclophosphamide and prednisone. Blood 46, 1–9 (1975)
Maldonado, J.E., Brown, A.L., Jr., Bayrd, E.D., Pease, G.L.: Ultrastructure of the myeloma cell. Cancer 19, 1613–1627 (1966)
Osserman, E.F., Takatsuki, K.: Clinical and immunochemical studies of four cases of heavy (Hγ 2) disease. Am. J. Med. 37, 351–373 (1964)
Sabatini, D.D., Bensch, K., Barrnett, R.J.: Cytochemistry and electron microscopy. The preservation of cellular ultrastructure and enzymatic activity by aldehyde fixation. J. Cell Biol. 17, 19–58 (1963)
Seligmann, M.: Heavy chain diseases. Rev. Europ. Études Clin. et Biol. 17, 349–355 (1972)
Stramignoni, A.: Diagnosi istologica e classificazione dei linfomi maligni. Giorn. Accad. Med. di Torino 139, 3–24 (1976)
Tsuji, T.: Heavy chain (Fc fragment) disease. Immunological reactions of the first case in Japan and the incorporation of 14-C-amino-acids into the protein vitro. Acta Haemat. Jap. 33, 89–99 (1970)
Tsunoda, R., Terashima, K., Takahashi, K., Kojima, M.: An ultrastructural study with the enzymelabeled antibody technique on immunoglobulin-containing cells in human tonsils, expecially in germinal centers. Acta Path. Jap. 28, 53–75 (1978)
Wager, O., Räsänen, J.A., Lindberg, L., Mäkelä, V.: Two cases of IgG heavy-chain disease. Acta Pathol. Microbiol. Scand. 75, 350–352 (1969)
Westin, J., Eyrich, R., Falsen, E., Lindholm, L., Lundin, P., Lönnorth, I., Weinfeld, A.: Gamma heavy chain disease. Reports of three patients. Acta Med. Scand. 192, 281–292 (1972)
Woods, R., Blumenschein, G.R., Terry, W.D.: A new type of human gamma heavy chain disease protein. Immunochemical and physical characteristics. Immunochemistry 7, 373–381 (1970)
Zawadzki, Z.A., Benedek, T.G., Ein, D., Easton, J.M.: Rheumatoid arthritis terminating in heavychiin disease. Ann. Intern. Med. 70, 335–347 (1969)
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This work has been partially supported by the Consiglio Nazionale delle Ricerche (C.N.R.), Centro di Immunogenetica ed Istocompatibilitá, Torino
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Stramignoni, A., Carbonara, A., Paolino, W. et al. G1 heavy chain disease clinicopathological, ultrastructural and immunochemical study of a new case. J Cancer Res Clin Oncol 96, 93–103 (1980). https://doi.org/10.1007/BF00412900
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DOI: https://doi.org/10.1007/BF00412900